Pheochromocytoma: seconds from disaster

1C.I.Parhon National Institute of Endocrinology, Bucharest, Romania; 2Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.

A 50year-old female with a 2 year-history of hypertension and diabetes was admitted to a county hospital, where treatment with clonidine and beta-blockers was started, without success. Abdominal CT showed a large left adrenal tumour, and her initial blood pressure (BP) was over 300/170 mmHg, which dropped to 80/30 mmHg. She was transferred for evaluation of a possible pheochromocytoma (PHEO). At admission her BP was 260/160 mmHg, heart rate (HR) 120 bpm, with anxiety, diaphoresis, cyanosis and pallor, but no retrosternal pain. ECG showed sinus tachycardia with ST segment depression and deep, symmetric T wave inversion (1014 mm) in V2-V6 leads and inverted T waves in I, II, aVL leads suggesting acute ischemia-lesion; QT interval was prolonged. Blood samples for cathecholamine metabolites were difficultly taken due to vasoconstriction. After alpha-beta blockers, calcium channels blockers and nitroglycerine her systolic BP oscillated between 300 and 40 mmHg; after one hour more, she developed persistent severe hypotension. Despite a perfusion with cortisone was started, after a small improvement, her BP collapsed, the visual acuity decreased and she became unresponsive, HR=120 bpm. She was transferred under ECG monitorisation and oxygen to the emergency hospital. On the way, she became responsive but at arrival she repeated the vascular collapse. A central catheter was placed and she received 2 litres saline and 400 mg cortisone in less than one hour, allowing filling of the vascular space and better BP control; she was admitted to the ICU under continuous monitorisation and oxygen. Meanwhile, very high levels of catecholamine metabolites confirmed PHEO: metanephrine =3365 pg/ml (N 1090), normetanephrine =4943 pg/ml (N 15100) and chromogranin A=1439 pg/ml (N 40100). After two weeks preoperative preparation with phenoxybenzamine, calcium channel blockers and nitroglycerine for control of the BP and HR, and restoration of blood volume, the patient underwent successful left adrenalectomy by open surgery with removal of a 15/15/12 cm left medulla tumour. Pathology and immunohistochemistry certified benign PHEO. Postoperatively catecholamine metabolites normalized (MN=10 pg/ml, NMN=30 pg/ml, CrA=40 pg/ml) as well as ECG and glucose metabolism. In addition, Takotsubo cardiomyopathy induced by PHEO resolved.

Our case illustrates how some PHEO can be at seconds from disaster, but with a happy end. Intensive management and multidisciplinary team is compulsory.